Objective: To explore the program of preoperative evaluation, preoperative preparation and separation operation of pygopagus conjoined twins.
Methods: Clinical data of one case of pygopagus (male, gestational age 37 weeks, uterine-incision delivery, 3.5 months old; twins of triplets; total body weight 8.0 kg. The twins have self-governed extremities, anus, penis. Four limbs can move independently). Separated successfully were analyzed. The auxiliary examination of X-rays, MRI, ultrasound, CT and 3D reconstruction, sensory evoked potential showed that there were process hypoplasia and lamina bifid of lumbosacral vertebrae below L3. Dural sac connected below L3 and conus medullaris located at L3. There were no conjunction of spinal cord and cauda equine. The decision of direct suture of dural sac was made by preoperative evaluation through measurement of circumference and area of conjoined dural sac. The separation surgery program was decided through team debate and sham operation. Separation operation was performed under the general anaesthesia. Crossing V-shaped skin flap was used to cover the wound surface. The dura of conjoined twins were sutured directly. The tension of skin flap was normal postoperative with continuing negative pressure drainage under the flap and pressure sterilized dressing.
Results: Successful separation of pygopagus conjoined twins was achieved. Lower extremities movements of separated twins were normal 6 h after operation. The drainage was removed 1 day postoperative, blood supplies of skin flap were normal. There were no complications of infection, cerebrospinal fluid leakage and neurological deficit. The healing of the skin flap was good. With 6 months follow-up, the growth and neurological function were normal.
Conclusion: Consummate preoperative preparation, accurate preoperative investigations, meticulous operative management, careful postoperative administration and good team cooperation are the keys to successful separation of pygopagus conjoined twins.